Dermatofibrosarcoma Protuberans
What is
it?
Dermatofibrosarcoma protuberans is a rare type of
malignant tumor that occurs in the dermis, the second
layer of the skin.
Who gets
it?
Dermatofibrosarcoma protuberans is uncommon, but
occurs slightly more frequently in males than females.
It can occur at any age, but is more likely to occur
in adults.
What
causes it?
The cause
of dermatofibrosarcoma protuberans is unknown.
What are
the symptoms?
Dermatofibrosarcoma protuberans is a slow-growing
tumor that is most commonly seen on the trunk of the
body (chest, abdomen, and back), but can also occur on
the arms, legs, head, neck, hands, or feet. The tumor
begins as a very small bump on the surface of the
skin, sometimes having a reddish-brown or reddish-blue
color. The tumor then undergoes a period of very rapid
growth where the many small growths in the dermis form
a larger, more noticeable mass. This mass swells and
bulges outward, hence the term “protuberans.” The area
becomes tender and may bleed because the top layer of
the skin is stretched so tight over the mass.
Dermatofibrosarcoma protuberans usually does not
spread to other parts of the body (metastasize).
How is it
diagnosed?
Because the
initial growth phase is so slow, it can be a few years
before the tumor is noticeable and causes enough
discomfort for the patient to seek a diagnosis. To
diagnose dermatofibrosarcoma protuberans, a doctor
will remove a sample of the affected tissue and send
it to a laboratory for examination under a microscope.
This is called a biopsy. The laboratory can determine
whether the cells and growth pattern in the sample are
characteristic of dermatofibrosarcoma protuberans.
What is
the treatment?
Dermatofibrosarcoma protuberans has commonly been
treated with wide excision, which means the tumor and
a large amount of the tissue surrounding it are
removed. Mohs’ microscopically controlled surgery
(also called Mohs’ micrographic surgery) is another
option that has been very effective. This procedure
involves removing the tumor, then continuing to remove
the edges of tissue around the tumor site. After each
bit of tissue is removed, it is examined under a
microscope for signs of malignant cells. The doctor
continues removing tissue around the tumor site until
there is no longer any sign of malignant cells. How
the area is closed after wide excision or Mohs’
surgery depends upon how much tissue must be removed.
Dermatofibrosarcoma protuberans has a high recurrence
rate because the tumor has many projections beyond the
main mass that may be left behind when the tumor is
removed. However, Mohs’ surgery has proven very
effective in reducing the recurrence rate.
Chemotherapy is not an effective treatment because the
tumor grows so slowly. Radiation therapy may be
recommended for some patients.
Self-care
tips
If you have
been diagnosed with dermatofibrosarcoma protuberans,
it is important to maintain your follow-up
appointments after treatment. If the tumor is going to
recur, it will most likely happen during the first
three years after surgery.
This information has been designed as a comprehensive and quick reference
guide written by our health care reviewers. The health information written
by our authors is intended to be a supplement to the care provided by your
physician. It is not intended nor implied to be a substitute for
professional medical advice.
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